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Von Willebrand disease

Marika Pikta, Galina Zemtsovskaja, Hector Bautista, Georges Nouadje, Timea Szanto, Margus Viigimaa, Valdas Banys
BACKGROUND: The von Willebrand factor (VWF) multimer test is required to correctly subtype qualitative type 2 von Willebrand disease (VWD). The current VWF multimer assays are difficult, nonstandardized, and time-consuming. The purpose of this study was to evaluate the clinical utility of the commercial VWF multimer kit by Sebia (Lisses, France), an electrophoresis technique yielding same-day results. METHODS: Ten healthy volunteer plasma samples, in-house reference plasma (IRP) and commercial normal plasma (CNP) samples, 10 plasma samples from patients with a known VWD type, 1 hemophilia A plasma sample, and 7 external quality assurance (EQA) samples were analyzed using the commercial VWF multimer kit...
February 17, 2018: Journal of Clinical Laboratory Analysis
Christine Demers, Christine Derzko, Michele David, Joanne Douglas
OBJECTIVE: The prevalence of bleeding disorders, notably von Willebrand disease (vWD), among adult women with objectively documented menorrhagia is consistently reported to be 10% to 20% and is even higher in adolescents presenting with menorrhagia. OPTIONS: Diagnostic tools and specific medical and, where appropriate, surgical alternatives to management are reviewed and evidence-based recommendations presented. EVIDENCE: A MEDLINE search of the English literature between January 1975 and November 2003 was performed using the following key words: menorrhagia, uterine bleeding, pregnancy, von Willebrand, congenital bleeding disorder, desmopressin/DDAVP, tranexamic acid, oral contraceptives, medroxyprogesterone, therapy, hysterectomy, anesthesia, epidural, spinal...
February 2018: Journal of Obstetrics and Gynaecology Canada: JOGC, Journal D'obstétrique et Gynécologie du Canada: JOGC
N V Khatri, B Patel, D R Kohli, S S Solomon, K Bull-Henry, C M Kessler
INTRODUCTION: Lenalidomide is a thalidomide analog with anti-angiogenic properties. Previous case reports suggest its efficacy in preventing gastrointestinal bleeding (GIB) secondary to angiodysplasia (AD) in hereditary haemorrhagic telangiectasia and potentially in reversing AD. We present the first case series to explore lenalidomide as a treatment for AD-related GIB in patients with von Willebrand disease (VWD). METHODS: A retrospective chart review was conducted to include patients with VWD, who were evaluated from 2010 to 2013 and who had received lenalidomide to treat recurrent GIB secondary to AD...
February 15, 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
Marina Panova-Noeva, Andreas Schulz, Natalie Arnold, Iris M Hermanns, Jürgen H Prochaska, Dagmar Laubert-Reh, Henri M Spronk, Maria Blettner, Manfred Beutel, Norbert Pfeiffer, Thomas Münzel, Karl J Lackner, Hugo Ten Cate, Philipp S Wild
BACKGROUND: The advancements in cancer treatment and detection of early cancer have resulted in steady increase of cancer survivors over the years. However, due to long term toxic effects of chemotherapy and radiotherapy, the incidence of cardiovascular disease (CVD) is increasing in survivors. OBJECTIVES: This study investigates traditional cardiovascular risk factors (CVRFs), inflammation and coagulation profile in long-term cancer survivors (cancer diagnosis ≥ five years) from a large adult population-based study sample...
February 12, 2018: Journal of Thrombosis and Haemostasis: JTH
Nicholas Bercovici, Kristen Russomanno, Frank Santoro, Christopher Scola, Michael Murphy, Zendee Elaba
Injection drug abuse (IDA) is known to cause a spectrum of systemic and cutaneous complications. Despite the increasing incidence of IDA around the world, there is a paucity of literature discussing cutaneous complications from a dermatopathologic perspective. We present a case of a 35-year-old male with a complex medical history of Von Willebrand disease, Behcet's disease and diverticular disease. Following a sigmoidectomy/colostomy for diverticular perforation, he presented with fever and an indurated right arm displaying livedoid purpura...
February 12, 2018: Journal of Cutaneous Pathology
V Nummi, R Lassila, L Joutsi-Korhonen, E Armstrong, T Szanto
INTRODUCTION: Diagnosis of von Willebrand disease (VWD) is challenging, particularly for type 1. The current diagnostic guidelines emphasize simultaneous bleeding symptoms and von Willebrand factor (VWF) levels of <30-40 IU/dL. Historical diagnoses require updated evaluation. We assessed the accuracy of past VWD diagnoses in our comprehensive care center with the standardized bleeding score (BS) and central laboratory analysis, focusing on VWF-dependent platelet functions in whole blood...
February 10, 2018: International Journal of Laboratory Hematology
Karl C Desch
Von Willebrand factor (VWF) is a multimeric plasma glycoprotein that plays a central role in the initiation of blood coagulation. Through interactions between its specific functional domains, the vascular wall, coagulation factor VIII, and platelet receptors, VWF maintains hemostasis by binding to platelets and delivering factor VIII to the sites of vascular injury. In the healthy human population, plasma VWF levels vary widely. The important role of VWF is illustrated by individuals at the extremes of the normal distribution of plasma VWF concentrations where individuals with low VWF levels are more likely to present with mucocutaneous bleeding...
2018: F1000Research
Hong Jin, Yifei Chen, Bilei Wang, Yi Zhu, Long Chen, Xiqiong Han, Genshan Ma, Naifeng Liu
BACKGROUND: Brain-derived neurotrophic factor (BDNF) is a neurotrophin involved in angiogenesis and maintenance of endothelial integrity. Whether circulating BDNF levels are associated with von Willebrand factor (vWF) levels, which are indicators of endothelial dysfunction is not known. This study investigated the association between plasma BNDF and vWF levels and whether these biomarkers could predict cardiovascular events at a 12-month follow-up in patients with stable coronary artery disease (CAD)...
February 6, 2018: BMC Cardiovascular Disorders
Candice M Baldeo, Candido E Rivera, Han W Tun, Prakash Vishnu
von Willebrand disease (VWD) is a common bleeding disorder caused by defective or low levels of von Willebrand factor (VWF). Although most cases of VWD are caused by genetic mutations, some are acquired due to various disease states. In managing VWD, the aim is to normalize plasma levels of both VWF and factor VIII (FVIII), as this aids in hemostasis. Desmopressin usually corrects VWF level in type 1 VWD by inducing the release of endogenous VWF. In cases where desmopressin is ineffective or cannot be used, transfusion of virally inactivated, plasma-derived VWF/FVIII concentrate or infusion of recombinant VWF (Vonvendi) is indicated...
2018: Journal of Blood Medicine
Joseph L Blackshear
Gastrointestinal bleeding with severe aortic stenosis was originally described in the 1950s by Heyde, although for years, the association was debated. Further discovery of mechanisms and the ubiquity and severity of acquired von Willebrand syndrome in the left ventricular assist device therapy have removed any doubts. At this time, gastrointestinal bleeding from intestinal angiodysplasia in patients with turbulence-related proteolysis of the highest molecular weight multimers of von Willebrand factor is now known to occur in patients with aortic stenosis, and also subaortic obstruction and associated mitral insufficiency in hypertrophic cardiomyopathy, isolated mitral and aortic insufficiency, endocarditis, and in patients with prosthetic valve dysfunction, either from stenosis or insufficiency...
February 3, 2018: Current Treatment Options in Cardiovascular Medicine
Eric Manderstedt, Christina Lind-Halldén, Stefan Lethagen, Christer Halldén
von Willebrand factor (VWF) levels in healthy individuals and in patients with type 1 von Willebrand disease (VWD) are influenced by genetic variation in several genes, e.g. VWF, ABO, STXBP5 and CLEC4M. This study aims to screen comprehensively for CLEC4M variants and investigate their association with type 1 VWD in the Swedish population. In order to screen for CLEC4M variants, the CLEC4M gene region was re-sequenced and the polymorphic neck region was genotyped in 106 type 1 VWD patients from unrelated type 1 VWD families...
2018: PloS One
J Gebhart, S Hofer, S Panzer, P Quehenberger, R Sunder-Plassmann, G Hoermann, E Eigenbauer, H Haslacher, S Kepa, P A Kyrle, S Eichinger, P Knöbl, L Eischer, C Mannhalter, C Ay, I Pabinger
INTRODUCTION: Data on clinical characteristics and the prevalence of underlying coagulopathies in patients with mild-to-moderate bleeding disorders (MBDs) are scarce. AIM: We established the Vienna Bleeding Biobank (VIBB) to characterize and thoroughly investigate Austrian patients with MBDs. RESULTS: Four hundred eighteen patients (female = 345, 82.5%) were included. A platelet function defect (PFD) was diagnosed in 26 (6.2%) and a possible PFD in 30 (7...
February 1, 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
Karthik Suresh, Laura Servinsky, Haiyang Jiang, Zahna Bigham, Xin Yun, Corinne Kliment, John C Huetsch, Mahendra Damarla, Larissa A Shimoda
Pulmonary arterial hypertension (PAH) is a lethal disease characterized by elevations in pulmonary arterial pressure, in part due to formation of occlusive lesions in the distal arterioles of the lung. These complex lesions may comprise multiple cell types, including endothelial cells (ECs). To better understand the molecular mechanisms underlying EC dysfunction in PAH, lung microvascular endothelial cells (MVECs) were isolated from normoxic rats (N-MVEC) and rats subjected to SU5416 plus hypoxia (SuHx), an experimental model of PAH...
February 1, 2018: American Journal of Physiology. Lung Cellular and Molecular Physiology
Rebecca Kruse-Jarres, Jill M Johnsen
Type 2B von Willebrand disease (VWD) is an inherited bleeding disorder caused by changes in von Willebrand factor (VWF) that enhance binding of VWF to GPIb on platelets. Although this disorder is seemingly well defined due to this single molecular defect, in reality type 2B VWD is a clinically heterogeneous disorder that can be difficult to identify and manage. Diagnostic criteria include a history of mucocutaneous bleeding, laboratory studies showing enhanced VWF binding of platelets and/or a 2B VWD genetic variant, and a family history consistent with autosomal dominant inheritance...
January 29, 2018: Blood
Myriam Ferrari, Federico Galvanin, Massimiliano Barolo, Viviana Daidone, Roberto Padrini, Fabrizio Bezzo, Alessandra Casonato
A reduced von Willebrand factor (VWF) synthesis or survival, or its increased proteolysis, alone or in combination, contributes to the development of von Willebrand disease (VWD).We describe a new, simple mechanistic model for exploring how VWF behaves in well-defined forms of VWD after its 1-desamino-8-D-arginine vasopressin (DDAVP)-induced release from endothelial cells. We aimed to ascertain whether the model can consistently predict VWF kinetic changes. The study involved 9 patients with VWD types Vicenza (a paradigmatic form with a reduced VWF survival), 8 type 2B, 2 type 2A-I, 1 type 2A-II (associated with an increased VWF proteolysis), and 42 normal controls, whose VWF levels were measured after a 24-hour-long DDAVP test...
February 2018: Thrombosis and Haemostasis
Xiaoxi Pan, Yun Yun Gong, Ya Xu, Robert A S Ariens, Michael N Routledge
BACKGROUND:  Particulate matter contained in ambient air pollution has been associated with cardiovascular diseases in several epidemiological studies. OBJECTIVE:  The aim of this study was to investigate the potential for urban particulate matter to induce changes in clot structure through interaction with vascular endothelial cells. METHODS:  We examined the structure of clots formed on human umbilical vascular endothelial cells that had been treated with various types of particles versus those formed on untreated cells...
February 2018: Thrombosis and Haemostasis
Magdalena Budzyń, Maria Iskra, Wojciech Turkiewicz, Zbigniew Krasiński, Bogna Gryszczyńska, Magdalena Paulina Kasprzak
BACKGROUND: Although the endothelial dysfunction is considered to be implicated in the pathogenesis of chronic venous insufficiency (CVI) the endothelial status in patients with venous disorders is still not fully evaluated. Therefore the aim of the study was to measure the concentration of selected markers of endothelial dysfunction: von Willebrand factor (vWf), soluble P-selectin (sP-selectin), soluble thrombomodulin (sTM) and soluble VE-cadherin (sVE-cadherin) in CVI women who constitute the most numerous group of patients suffering from venous disease...
2018: PloS One
Naamah L Zitomersky, Melanie Demers, Kimberly Martinod, Maureen Gallant, Stephen M Cifuni, Amlan Biswas, Scott Snapper, Denisa D Wagner
Background: Inflammatory bowel disease (IBD) affects 1.6 million people in the United States. IBD is associated with an increased risk of thrombosis, which rises with disease activity. The pathogenesis of IBD and its increased thrombotic risk is not completely understood. Ultra large von Willebrand factor (ULVWF) multimers are secreted from activated endothelium, leading to recruitment of platelets and leukocytes. A disintegrin and metalloproteinase with thrombospondin type I repeats motif 13 (ADAMTS13) cleaves highly adhesive ULVWF into smaller, less bioactive, multimers, releasing them into circulation...
June 2017: TH Open: Companion Journal to Thrombosis and Haemostasis
Stephane Fournier, Florine Guenat, Anne Fournier, Lorenzo Alberio, Olivier Bonny, Debora Bertaggia Calderara, Daniel Bardy, Nathalie Lauriers, Brahim Harbaoui, Pierre Monney, Patrizio Pascale, Eric Eeckhout, Olivier Muller
Aims: The circadian variation of platelet aggregation is well demonstrated. However, whether this has an impact on antiplatelet inhibition therapy is poorly documented. We aimed to observe whether ticagrelor-induced platelet inhibition follows a circadian rhythm. Methods and Results: The study included 25 healthy volunteers (11 female;14 male). Blood samples were collected every 4 hours. Ticagrelor was added in vitro at a concentration that provided 50% inhibition of the maximum response using the VerifyNow System Platelet Reactivity Test® thus avoiding any bias induced by circadian gastro-intestinal absorption...
January 23, 2018: European Heart Journal. Cardiovascular Pharmacotherapy
Lisa Epping, Wolfgang Miesbach, Katrin Nickles, Peter Eickholz
BACKGROUND: Von Willebrand disease (VWD) is the most common inherent bleeding disorder. Gingival bleeding is a frequently reported symptom of VWD. However, gingival bleeding is also a leading symptom of plaque-induced gingivitis and untreated periodontal disease. In type 1 VWD gingival bleeding was not increased compared to controls. Thus, this study evaluated whether type 2 and 3 VWD determines an increased susceptibility to gingival bleeding in response to the oral biofilm. METHODS: Twenty-four cases and 24 controls matched for age, sex, periodontal diagnosis, number of teeth and smoking were examined hematologically (VWF antigen, VWF activity, factor VIII activity) and periodontally (Gingival Bleeding Index [GBI]), bleeding on probing [BOP], Plaque Control Record [PCR], periodontal inflamed surface area [PISA], vertical probing attachment level)...
2018: PloS One
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